A new score for life-threatening ventricular arrhythmias and sudden cardiac death in adults with transposition of the great arteries and a systemic right ventricle.

Autor:	Ladouceur M; Centre de référence des Malformations Cardiaques Congénitales Complexes, M3C, Adult Congenital Heart Disease Unit, Hôpital Européen Georges Pompidou, APHP, Paris Cité University, Paris 75015, France.; Centre de Recherche Cardiovasculaire de Paris, INSERM U970, 20 rue Leblanc, Paris 75015, France., Van De Bruaene A; Division of Congenital and Structural Cardiology, University Hospitals Leuven, and Catholic University Leuven, Leuven, Belgium., Kauling R; Department of Cardiology, Thoraxcenter, ErasmusMC, University Medical Center Rotterdam, Rotterdam, The Netherlands., Budts W; Division of Congenital and Structural Cardiology, University Hospitals Leuven, and Catholic University Leuven, Leuven, Belgium., Roos-Hesselink J; Department of Cardiology, Thoraxcenter, ErasmusMC, University Medical Center Rotterdam, Rotterdam, The Netherlands., Albert SV; Paediatric Cardiology and ACHD Unit, Ramon y Cajal University Hospital, Madrid, Spain., Perez IS; Paediatric Cardiology and ACHD Unit, Ramon y Cajal University Hospital, Madrid, Spain., Sarubbi B; Adult Congenital Heart Disease Unit, AORN dei Colli-Monaldi Hospital, Naples, Italy., Fusco F; Adult Congenital Heart Disease Unit, AORN dei Colli-Monaldi Hospital, Naples, Italy., Gallego P; Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitario Virgen del Rocio, Instituto de BioMedicina de Sevilla (IBIS) and CIBERCV, Sevilla, Spain., Rodriguez-Puras MJ; Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitario Virgen del Rocio, Instituto de BioMedicina de Sevilla (IBIS) and CIBERCV, Sevilla, Spain., Bouchardy J; Service of Cardiology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.; Service de Cardiologie, Département de Médecine, Hôpitaux Universitaires de Genève, 1205 Genève, Switzerland., Blanche C; Service de Cardiologie, Département de Médecine, Hôpitaux Universitaires de Genève, 1205 Genève, Switzerland., Rutz T; Service of Cardiology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland., Prokselj K; Department of Cardiology, University Medical Centre Ljubljana, Ljubljana, Slovenia.; Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia., Labombarda F; Cardiology Department, CHU de Caen, Caen, France.; UNICAEN, EA 4650 Caen, France., Iserin L; Centre de référence des Malformations Cardiaques Congénitales Complexes, M3C, Adult Congenital Heart Disease Unit, Hôpital Européen Georges Pompidou, APHP, Paris Cité University, Paris 75015, France.; Centre de Recherche Cardiovasculaire de Paris, INSERM U970, 20 rue Leblanc, Paris 75015, France., Wong T; Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.; National Heart and Lung Institute, Imperial College School of Medicine, London, UK., Gatzoulis MA; Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.; National Heart and Lung Institute, Imperial College School of Medicine, London, UK.
Jazyk:	angličtina
Zdroj:	European heart journal [Eur Heart J] 2022 Jul 21; Vol. 43 (28), pp. 2685-2694.
DOI:	10.1093/eurheartj/ehac288
Abstrakt:	Aims: To investigate the incidence of major adverse ventricular arrhythmias and related events (MAREs) and to develop a stratification tool predicting MAREs in adults with a systemic right ventricle (sRV). Methods and Results: In a multicentre approach, all adults (≥16 years old) with a sRV undergoing follow-up between 2000 and 2018 were identified. The incidence of MAREs, defined as sudden cardiac death, sustained ventricular tachycardia, and appropriate implantable cardioverter-defibrillator (ICD) therapy, was analysed. The association of MAREs with clinical, electrical, and echocardiographic parameters was evaluated. A total of 1184 patients (median age 27.1 years; interquartile range 19.9-34.9 years; 59% male; 70% with atrial switch repair for D-transposition of the great arteries) were included. The incidence of MAREs was 6.3 per 1000 patient-years. On multivariate analysis, age, history of heart failure, syncope, QRS duration, severe sRV dysfunction and at least moderate left ventricular outflow tract obstruction were retained in the final model with a C-index of 0.78 [95% confidence interval (CI) 0.72-0.83] and a calibration slope of 0.93 (95% CI 0.64-1.21). For every five ICDs implanted in patients with a 5-year MARE risk >10%, one patient may potentially be spared from a MARE. Conclusion: Sudden cardiac death remains a devastating cause of death in a contemporary adult cohort with a sRV. A prediction model based on clinical, electrocardiographic, and echocardiographic parameters was devised to estimate MARE risk and to identify high-risk patients who may benefit from primary prevention ICD implantation. (© The Author(s) 2022. Published by Oxford University Press on behalf of European Society of Cardiology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)
Databáze:	MEDLINE
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